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Chaerul Anwar MD., RD Robin H. Wibowo MD., and Muhammad Munawar MD, PhD,.
Case report
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A 84-year-old man, was admitted to our emergency department on 10 May 2008
because of sudden onset of diaphragmatic stimulation, twitching of the right upper
extremity and severe bradycardia due to loss capture of his pacemaker (figure 1). No
history of having stroke, but he has suffered from dementia over the last 2 years. He
had been complaining of fatigue and light headiness for the past 6 months.
When he was at our emergency department, physical examination revealed his arterial
blood pressure was 120/70 mmHg and his pulse rate was 34/min. Cardiac auscultation
findings were normal. A pulsation in his abdomen due to diaphragmatic stimulation
was observed. The right extremity twitching was also observed. Programming
pacemaker
confirmed there was insulation leakage and the impedance was very low (< 100 ohm)
leading to battery depletion. Neither sensing nor pacing was possible (figure 1).
On
chest X-rays taken at our hospital, the tip of ventricular lead was in the distal cephalic
vein, and there was coaxial twisting of the lead around the pacemaker generator
(Figure 2).
The patient was brought into cathlab, and temporary pacemaker was inserted. The
2
damaged lead was removed without any complication. Because both lead and pulse
generator are damage (figure 3), a new VVI pulse genitor set was implanted without
any difficulties. The lead was positioned at the mid septal of the right ventricle. The
new pocket was made intra-pectoral muscle and pulse generator was sutured to
surrounding tissues with un-absorbable suture. On 12 months follow-up, he is well
and the pacemaker was having normal function.
Discussion
Leaving aside injuries and iatrogenic conditions, several syndromes of lead macro-
dislodgement have been described and cover the usual causes of lead dislodgement, namely,
twiddler, reel, and ratchet syndromes.2–4 Although the problem is relevant, the terminology is
confusing, perhaps because these terms were originally taken from individual case reports in
the literature. Identical cases have been defined and classified differently by the authors and,
conversely, different cases have been classified as identical. Some cases initially described as
twiddler syndrome are actually forms of reel syndrome, and some cases described as either of
these entities are actually cases of ratchet syndrome.5,6 Therefore, it would be appropriate to
provide precise definitions for each of the potential mechanisms, in order to enhance our
understanding of the mechanisms involved in each patient and to identify predictors of the
problem,their consequences, and the frequency of actual presentation of each one.
Below we present a proposal for the precise definition and classification of these lead macro-
dislodgement syndromes
Twiddler syndrome: Twiddler syndrome can be defined as lead retraction and dislodgement
due to device generator rotation over the axis defined by the lead. Although external
manipula- tion by the patient may make it easier for this to occur, it is not a necessary
condition. As the generator rotates, the lead twists over itself, giving it a characteristic and
definitive appearance (Fig. 2).
Reel syndrome: Reel syndrome can be defined as lead retraction and dislodgement due to
generator rotation over its sagittal axis, which causes lead reeling above or below the
generator. Because of the mechanism involved in both twiddler and reel syndrome, all leads
would be affected to some extent, in case of several leads.
Prior to surgical revision of the system in the case of lead dislodgement, simple visualization
of the lead(s) and radiographic visualization of the generator position, along with a
comparison to the original implant position, will provide an approximate identification of the
mechanism involved. For cases in which the evidence is not definitive, either because the
system uses only 1 lead and there are no signs of any mechanism (eg, twisting) or because the
generator is in its normal position and there are no lead abnormalities, it would be preferable
to use the term ‘‘lead macro-dislodgement’’ alone.
Conclusion
With better understanding the classification and management of the macro dislodgement
characteristic may be helpful for the clinician to implant the device and would allow early
characterization of complication in patients with implantable electronic cardiac devices.
Twiddler’s syndrome Reel syndrome
Mechanism Rotation of the Rotation of the
pacemaker generator on pacemaker generator on
its long axis its transverse axis
(rachet mechanism)
X ray Tangling of lead or leads Lead or leads rolling
around pacemaker around pacemaker
generator generator
Lead consequences Lead damage may occur No damage of the lead
either lead fracture or
insulation leakage.
Sometimes with depleted
battery
Procedure of treatment Replace with a new lead Reposition of the lead
and maybe pacemaker
generator
Occurence Possibly within a year Within a month
References
Arias, Miguel A. et al. Terminology Management for Implantable Cardiac Electronic Device
Lead Macro-Dislodgement. Rev Esp Cardiol. 2012;65(7):669–677. Document downloaded from
http://www.revespcardiol.org, day 05/10/2014